Split cord malformations (SCM) are increasingly being recognized as one of the causes of tethered cord syndrome. Renaming them as SCMs has reduced the conflict in terminology, though the pathogenesis of these complex anomalies still remains controversial. The diagnosis has become easy and certain with the advent of magnetic resonance imaging (MRI). It is necessary to screen the entire spine in order to recognize all the associated anomalies and to plan the management strategies. Aim: To study the role of prophylactic surgery in patients with SCM. Settings and Design: Retrospective clinical analysis. Methods and Material: In a series of 217 children treated with spinal dysraphism from 1994 to 2004 at Manipal Institute for Neurological disorders (MIND), 38 had SCMs and the clinical presentation and surgical outcome was retrospectively analyzed.
Statistical analysis used Results: 34 children had isolated SCM, four children had other complex congenital anomalies. 23 children [type 1-18 (47%) and type 2-20 (53%)] were operated prophylactically. They maintained neurological stability after surgery. Among the children with pre-existing neurological deficits, stability was achieved in all and significant improvement in only 8%. Outcome was better in isolated SCM in compression to complex ones. Conclusion: We recommend surgery before the onset of neurological deficits. Surgical results are excellent with good microsurgical technique

Hyponatremia in acute neurological disorders - Is it always due to siadh ?

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Indira Jayakumar, S Ranjit, C Balasubramaniam

Hyponatremia in acute CNS diseases is often attributed to the Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH). Other causes may be in operation and may be overlooked. Aims: The objective of the study was to determine the etiology, evaluate treatment modalites and assess the outcome in children with an underlying acute neurologic disease who were hyponatremic. All these children were admitted to the Intensive Care Unit (ICU). Methods and Materials: This is descriptive hospital based retrospective chart review. Clinical indices of hydration, serum and urine sodium and osmolality were used in children to determine the cause of hyponatremia. In such of those who were hyponatremic, the cause of hyponatremia, treatment and outcome were assessed. Management of hyponatremia depended on etiology and severity of symptoms. Symptomatic patients had serum sodium raised by 3-5 mEq/l in order to control symptoms, following which a more gradual correction was carried out. Children with SIADH were fluid restricted while those with hyponatremic dehydration and Cerebral Salt Wasting (CSW) received supplemental saline and fluids. Results: Out of 1371 Pediatric Intensive Care Unit (PICU) admissions over a 30-month period, 385 (28%) had primary CNS disorders and of these, 58 were hyponatremic. The causes were SIADH in 19 (33%), hyponatremic dehydration in 16 (28%), drug-induced hyponatremia in 13 (22%) and CSW in 10 (17%) patients. About 10 of the 58 hyponatremic patients expired. All deaths were due to the severity of the underlying neurological condition. About 3 patients were hyponatremic at the time of death. Conclusion: The etiology of hyponatremia in acute CNS disease is multifactorial, and is not always due to SIADH. Careful evaluation and targeted therapy is required for the optimal management of these children

Surgery for frontoethmoidal encephalocoeles involves correction of the cosmetic deformity, hypertelorism, orbital dystopia, elongation of the nose and other stigmata of midline or lateral facial defects. The technical aspects of a single stage correction of this disorder is presented. The coexistence of Chiari malformation (type 1), mild ventriculomegaly with the nasoethmoidal encephalocoele was a rare association in the reported case.

'White cerebellum sign' is a classic yet uncommon radiological finding in severe pediatric head injury. We present a case of a 7-year-old child who had this finding on computed tomography. We also discuss the possible etiogenesis and review the relevant literature.

Intramedullary lipomas are rare tumours of the spinal cord. The account for about 1% of spinal neoplasms (Neurosurgery, 12, 1983, 460). Their association with a dysraphic state is well known and is common but their occurrence in the absence of a neural tube defect is very rare. They can occur anywhere along the length of the spinal cord but their extension into posterior cranial fossa is very rare. We are herewith reporting a case of intradural cervical spinal lipoma with extension into the posterior cranial fossa. The diagnosis was established by magnetic resonance imaging. The intraspinal portion was resected and the child is awaiting surgery for the intracranial part.

Variations of the anterior cerebral artery are well known. However, the infraoptic course of the ACA is a rare anomaly. We present a case report of a patient with such an anomaly with an associated ruptured ipsilateral ICA bifurcation aneurysm with its attendant management problems.